Congenital diaphragamatic hernia associated with aortic coarctation: a case report
© Okawada et al; licensee BioMed Central Ltd. 2008
Received: 06 November 2008
Accepted: 08 December 2008
Published: 08 December 2008
Congenital diaphragmatic hernia associated with cardiac anomalies is a major therapeutic challenge. We report a case of Congenital diaphragmatic hernia associated with coarctation of the aorta.
Up to 40% of patients with congenital diaphragmatic hernia (CDH) have associated anomalies. Of those, cardiac anomalies, the most common, are present in 63% . Treatment of CDH with cardiac anomalies is a major challenge for pediatric and cardiovascular surgeons. We report a case of CDH with aortic coarctation that was treated successfully.
The association of CDH with cardiovascular malformations has been well described, but therapies have changed over the years. Cohen et al reported on the influence of congenital heart disease on the survival of children with CDH and only 5 of 16 patients survived . CoA often coexists as a component of left ventricular hypoplasia or small left ventricle [3–6], which is almost universally fatal and is now increasingly recognized as the most commonly associated cardiac malformation associated with CDH . However, in cases of CDH with isolated CoA, the ultimate outcome is related to the extent of pulmonary hypoplasia and persistent pulmonary hypertension not the CoA, which is a correctable cardiac malformation. In the present case, cardiovascular and respiratory status were maintained using a combination of HFOV, NO, vasoactive agents such as PGE1 and catecholamines without ECMO. We guess that our good outcome depended on mild pulmonary hypoplasia and persistent pulmonary hypertension, although the tight preductal CoA and VSD in combination with a large PDA resulted in right to left shunting for post CoA aortic flow.
ECMO is generally indicated in severe cases of CDH. Interestingly, the accuracy of prenatal diagnosis of CDH is not matched by the accuracy of fetal EC for predicting associated cardiac malformations. The diagnosis of cardiac malformation is often made at failure of weaning from ECMO or at autopsy, despite apparently normal EC at birth [7, 8]. Prenatal and/or postnatal diagnosis of cardiac malformation by EC is essential for appropriate management of CDH with cardiac malformations including the decision whether or not to use ECMO.
In the present case, correction of CoA was performed seven days after CDH repair. The timing of surgery was determined by improvement in PPH, such as no need for NO, disappearance of TR, and increased lPA blood flow. EC is also indispensable for determining the proper timing of cardiovascular surgery.
Successful management of CDH associated with cardiac malformations largely depends on the severity of pulmonary hypoplasia and persistent pulmonary hypertension, and the maintainance of cardiovascular function. The early and appropriate diagnosis of cardiac anomalies and the appropriate timing of surgical intervention using EC improve the outcome of CDH with cardiac malformations.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Congenital Diaphragmatic Hernia
High Frequency Oscillatory Ventilation
Coarctation of the Aorta
Patent Ductus Arteriosus
Ventricular Septal Defect
Tricuspid valve Reflux
left Pulmonary Artery
Persistent Pulmonary Hypertension
Extracorporeal Membrane Oxygenation
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